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Disease Profile

Superior limbic keratoconjunctivitis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

-

ICD-10

H16.2

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

SLK; Theodores superior limbic keratoconjunctivitis; Theodores syndrome;

Categories

Eye diseases

Summary

Superior limbic keratoconjunctivitis (SLK) is a chronic and recurrent eye disease which affects the superior bulbar conjunctiva (the clear layer that covers the eyeball, over the sclera) and tarsal conjunctiva (the clear layer that lines the eyelids), as well as the superior limbic aspect of the cornea (the area above the cornea).[1][2] It is commonly found in women 20-70 years of age. The signs and symptoms include burning, redness and irritation and tend to develop slowly over a period of 1 to 10 years.[3] Vision usually remains intact. While the underlying cause of SLK remains unknown, it is believed that the condition is secondary to superior bulbar conjunctiva laxity. Factors inducing conjunctiva laxity include thyroid eye disease (usually hyperthyroidism), tight upper eyelids, and prominent globes.[1][2][3] A mimicking disorder has been encountered in soft contact lens (SCL) wearers, typically with exposure to thimerosal-preserved solutions.[2] Treatment of SLK may involve the use of various medications, surgery, or a combination of both.[4]

Symptoms

The symptoms of SLK develop slowly over 1 to 10 years and usually occur in both eyes (bilateral). Vision is rarely affected. The most common symptoms include burning, irritation and redness.[2][3] These symptoms may be more pronounced when looking up.[2]

Cause

The cause of SLK is unknown. One of the most common theories suggests that the development of SLK is related to laxity or a loosening of the superior bulbar conjunctiva (the clear layer that covers the eyeball, over the sclera), resulting in friction and inflammation (swelling) between the upper eyelid and the eyeball. However, like other theories previously proposed, such as infectious, immunogenic and allergic, this theory lacks sufficient and convincing evidence to explain all of the developments of the disease. SLK may be associated with thyroid abnormalities, particularly hyperthyroidism, but is also found in individuals with normal thyroid function.[2][3] In advanced cases, superior limbal stem cell deficiency syndrome may occur from the repeated microtrauma to the limbal stem cells.[5]

A different condition, known as Contact Lens-Induced Superior Limbic Keratoconjunctivitis (CLSLK) is an eye inflammatory condition with an almost identical presentation, but it is related to contact lens wear.[6]

Treatment

Many different methods have been used to try to manage SLK with varying levels of success. The most common treatment is large diameter contact lens wear.[3][7] Other options include:[2][4]

  • Silver nitrate (antiseptic) solution
  • Mast cell stabilizers (used to prevent or control allergic reactions)
  • Vitamin A preparations 
  • Cyclosporine A
  • Autologous (self) serum–derived drops
  • Botulinum injection to the overlying muscle

Surgical options include cryotherapy (use of cold to destroy abnormal tissue), conjunctival resection of the superior limbal conjunctiva, and thermal cautery of superior limbal conjunctiva, which may be curative.[3][7][4]

More detailed information related to the treatment of SLK can be accessed through Medscape Reference.

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Superior limbic keratoconjunctivitis. Click on the link to view a sample search on this topic.

References

  1. Kabat AG. Lacrimal occlusion therapy for the treatment of superior limbic keratoconjunctivitis. Optom Vis Sci. 1998; https://www.ncbi.nlm.nih.gov/pubmed/9798210. Accessed 10/6/2016.
  2. Oakman JH. Superior Limbic Keratoconjunctivitis. Medscape Reference. April 23, 2015; https://emedicine.medscape.com/article/1194578-overview. Accessed 10/6/2016.
  3. Papaliodis GN. Superior Limbic Keratoconjunctivitis. The Ocular Immunology and Uveitis Foundation. https://www.uveitis.org/docs/dm/superior_limbal_keratoconjunctivitis.pdf.
  4. Guillermo Mendoza, A., Rodríguez-García, A., Bernfeld, E, Feldman, B.. Superior limbic keratoconjunctivitis. American Academy of Ophthalmology. August 15, 2015; https://eyewiki.aao.org/Superior_limbic_keratoconjunctivitis.
  5. Chelala E, El Rami H, Dirani A, Fakhoury H & Fadlallah A. Extensive superior limbic keratoconjunctivitis in Graves’ disease: case report and mini-review of the literature. Clinical Ophthalmology (Auckland, NZ). 2015; 9:467-468. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4362972/.
  6. Contact Lens-Induced Superior Limbic Keratoconjunctivitis (CLSLK). Association of Optometric Contact Lens Educators. May, 2016; https://www.aocle.org/livingL/clslk.html.
  7. Fraunfelder FW. Liquid nitrogen cryotherapy of superior limbic keratoconjunctivitis. Am J Ophthalmol. 2009; https://www.ncbi.nlm.nih.gov/pubmed/18835475. Accessed 10/6/2016.

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